Question 1

General Infection

Accepted Answer

Since your child is on high doses of immunosuppression post-transplant, your child is at a high risk for infection. Because of this, it is important to monitor for infections and it is likely that you will be asked to take your child’s temperature daily after their transplant as well as be aware of his or her constitutional symptoms (e.g. fatigue, muscle aches).
Signs and Symptoms of Infection:
 • Oral temperature > 100.4 F (38 degrees C)
 • Chills or sweats
 • Cough
 • Shortness of breath
 • Sore throat
 • Earache
 • Sores or rashes in mouth or skin
 • Burning with urination
 • Cloudy or foul-smelling urine
 • Pain, redness, warmth on incision site
 • Wound or cut that won’t heal
 • Increased or watery diarrhea with foul smell
 • Redness, swelling, drainage around drainage tubes
Diagnosis: Depending on your child’s signs and symptoms will make the decision on what types of tests and procedures may be run. In general, tests may include:
 • Bloodwork
 • Urinalysis
 • Blood cultures
 • Stool cultures
 • CT scans of chest or abdomen
Treatment: Treatment is going to be based on the type of infection that your child has and whether or not your child is able to tolerate oral medications. Your child will be given either oral or IV antivirals, antibacterials, or antifungals depending on the organism your child is infected with.

Question 2

Urinary Tract Infection (UTI)

Accepted Answer

UTIs are common with immunosuppressed patients. These infections can involve the urethra, the bladder, and/or the kidneys. A variety of different organisms can cause these infections.
Signs and Symptoms:
 • Burning or pain with urination.
 • Feeling the urge to urinate more frequently and urgently (i.e. a child that was potty-trained may wet their pants and/or bed).
 • Foul smelling urine or blood in urine.
 • Pain in the lower back (if the kidney is infected).
 • Fever.
Prevention:
 • Avoid bubble baths.
 • Wear cotton underwear.
 • Drink plenty of water.
 • After toileting, always wipe front to back.
 • Encourage your child to pee often and avoid ‘holding it.’

Question 3

Chicken Pox (Varicella)

Accepted Answer

General information
 • Common childhood disease.
 • Can develop at any age, but occurs more frequently from ages 5 to 10 years.
 • Can be a serious illness for the immunosuppressed transplant recipient that can cause complications or death in severe cases.
 • Most transplant patients get a vaccine to prevent chicken pox before transplant. Transplant recipients cannot receive the vaccine after transplant as it is a live virus vaccine.
How it is spread:
 • Spread through the air or by close contact with someone who has the virus.
 • Contagious for 2 days before the rash occurs and until all the pox have developed a scab (usually 6 days after the rash appears).
 • If exposed, he or she could develop chickenpox anywhere from 14 to 21 days following exposure (the incubation period).
Signs and symptoms
 • Often begins with a fever and dry cough.
 • Splotchy rash may begin on the head and spread downward, or on the trunk and spread outward.
 • Rash consists of small, watery blisters with red rings around them.
 • A person with chickenpox feels very ill for a few days.
 • The rash may be altered in appearance because of the Varicella-Zoster Immune Globulin (V-ZIG) injection or immunosuppressant drugs.
Prevention
 • Please tell your friends and family, classmates, and teachers about the danger of chickenpox.
 • Ask them to call you if any child is exposed or gets chickenpox. We have found that most people are sympathetic to your situation and are willing to cooperate.
What to do if exposed?
 • Call your transplant coordinator immediately.
 • Your child will need to have a V-ZIG injection (varicella-zoster immunoglobulin) within 3 days of exposure (note- the injection may not prevent chickenpox, but it will lessen the severity of it).
If your child does get chicken pox even after the V-ZIG injection, your transplant team may require your child be admitted and started on acyclovir until the lesions are crusted over and the team feels your child is safe to be discharged.

Question 4

Shingles (Varicella)

Accepted Answer

General Information
 • Caused by a reactivation of the same virus that causes chickenpox (Varicella).
 • When the virus is reactivated, it will cause an outbreak of shingles (rash, blisters). You can get chicken pox when exposed to the drainage from these lesions.
Signs and Symptoms
 • One to three days before rash appears symptoms may include: pain, tingling, and burning on the side of the chest, neck, forehead, back, hip, or leg.
 • Rash and sores appear in clusters of blisters usually in a strip on one side of the body, torso, or face.
 • You will no longer be contagious when all lesions are scabbed over.
Treatment
 • Contact the transplant team immediately.
 • Treatment is the same as chicken pox above.

Question 5

Aspergillosis

Accepted Answer

Aspergillus is a common fungus (mold) that lives in decaying vegetable matter, soil, and dusty areas. For healthy people, it is of little concern, however, for those that are immunosuppressed it can cause a serious fungal infection.
Signs and Symptoms:
 • Fever and chills
 • Bloody cough
 • Shortness of breath
 • Chest pain
 • Joint pain
 • Nosebleed
 • Skin lesions
Diagnosis:
 • Chest CT or Chest X-ray
 • Sputum test
 • Tissue and blood tests
Treatment: If your child contracts the infection then your child’s team may treat your child with antifungal medications such as voriconazole or amphotericin B.

Question 6

Pneumocystis Jiroveci Pneumonia (aka pneumocystis carinii or PCP)

Accepted Answer

This is a serious infection that causes inflammation and fluid buildup in your child’s lungs. It is caused by a fungus called Pneumocystis jiroveci that spreads through the air and is very common. For normal, healthy people, this fungus is not harmful, but for transplant patients with a weakened immune system, it can cause a very serious infection. 
Signs and Symptoms:
 • Fever
 • Dry cough or wheezing
 • Shortness of breath
 • Fatigue
 • Chest pain while breathing
Diagnosis:
 • Fluid from lungs
 • Biopsy from lungs
 • Blood tests
Treatment: Patients are usually given prophylaxis (preventative treatment) with Bactrim (Sulfamethoxazole / Trimethoprim) three times per week. In the event that your child cannot tolerate the oral medication, then a monthly breathing treatment of Pentamidine may be suggested.

Question 7

Post-Transplant Complications Introduction

Accepted Answer

Complications are very common after an intestinal or multivisceral transplant. The positive thing is that your transplant team will be vigilant in monitoring your child for all complications and will be on top of treating your child if any complications do arise. Here is a sampling of potential of complications. This is not an all-inclusive list, nor does the explanation for each complication contain every possible symptom, treatment, or scenario.
 
If you are concerned that your child is experiencing a complication after his or her transplant, or are concerned about the possibility of complications, it is always best to contact your child’s transplant team directly. For a more detailed listing of complications, visit our adult patient section here.(javascript:void(0))

Question 8

Acute Cellular Rejection

Accepted Answer

This occurs when your child’s body detects the new intestine as foreign and, as a result, attacks the organ. Most patients will experience some form of rejection in the first 3-6 months post-transplant. This type of rejection can be classified as indeterminate, mild, moderate, or severe based on the histologic findings on your child’s endoscopic biopsy.
Signs and Symptoms:
 • Fever
 • Abdominal pain
 • Abdominal distention or swelling
 • Nausea/ vomiting
 • Increase in stoma output
 • Changes in the appearance of stool
 • Blood in stoma output
 • Change in appearance or color of stoma
 • Loss of appetite/ weight loss
 • Often causes no physical symptoms
Diagnosis: Diagnosis is made by taking a biopsy of your child’s intestine through the ileostomy with an endoscope. If your child is experiencing symptoms, this may prompt their transplant team to order an ileoscopy. On the other hand, since rejection often times does not cause symptoms, acute cellular rejection may be found on surveillance biopsies. The transplant team will rely on the pathology of the biopsy to confirm the diagnosis and severity of the rejection.
Treatment: Treatment ultimately depends on the severity of the rejection. Mild rejection may just require adjustment of your child’s immunosuppression medications and high doses of corticosteroids. Moderate to severe rejection may require heavier immunosuppression agents such as Thymoglobulin or Campath. Your child’s transplant team will determine the best course of treatment for your child based upon their immune status and biopsy results.

Question 9

Post-Transplant Lymphoproliferative Disorder (PTLD) and Epstein Barr Virus (EBV)

Accepted Answer

Epstein Barr Virus is a common virus. In healthy children, it usually results in the common manifestation known as ‘mono’ and the individual’s immune system is usually able to fight off the infection. However, in immunocompromised transplant patients, certain immune cells, are suppressed and are not able to keep the proliferating B cells in check. The Epstein Barr Virus that had been ‘asleep’ in your child’s immune cells called B cells ‘wakes up’ and begins to replicate. This viral replication goes uncontrolled by your child’s immune system and leads to PTLD.
Signs and Symptoms (mainly an overall feeling of being unwell):
 • Malaise, feeling of unease/ illness
 • Fatigue
 • Night Sweats
 • Fever
 • Weight Loss
 • Swollen lymph nodes
Diagnosis: Diagnosis of PTLD is based on looking at the entire clinical picture and considering many different tests. Your child’s transplant team will conduct a thorough physical and exam, especially taking note of any lymph node enlargement. They most likely will get extensive blood work, possible imaging studies (CT scan, PET scan, and/or MRI), urinalysis, and/or lumbar puncture. Depending upon your child’s unique clinical picture will determine what set of tests your team decides to order.
Treatment: The first line of treatment for PTLD is lowering immunosuppression. If this does not solve the problem your child’s team might consider starting your child on a medication called Rituximab which has been shown to have very positive results in treating PTLD. More refractory cases may be treated with chemotherapeutic agents or localized radiation. As always, your child’s transplant team will choose the treatment best suited to your child’s particular case.

Question 10

Graft vs. Host Disease (GVHD)

Accepted Answer

GVHD occurs in all types of transplants, however, it is of major concern to intestinal transplant due to the large amount of immune tissue that the intestine contains. GVHD results when the donor’s cells (the graft) view your child’s healthy cells (the host) as foreign and begin to attack and damage them.
Signs and Symptoms- can vary based on if the disease is local on the skin or effecting a particular organ:
 • Skin reactions: itching; red rash on the upper trunk, neck and feet; blisters on the palm, soles, and abdominal skin.
 • Mouth or tongue lesions.
 • GI manifestations: Diarrhea, nausea, vomiting.
 • Hepatitis, or liver inflammation: May not have symptoms, but may lead to jaundice or yellowing of the skin.
Diagnosis: Usually a tissue biopsy of the affected skin will be used to diagnose GVHD along with clinical picture and your child’s history. Blood tests will often be taken to help manage the condition and to check liver function. If gastrointestinal symptoms are present, an endoscopy may be ordered.
Treatment: In self-limited, mild skin cases of GVHD, no treatment may be necessary. In disseminated disease, steroid therapy is the treatment of choice along with adjustments in immunosuppression.

Question 11

Chylous Leak

Accepted Answer

This is a complication that results when the lymphatic system of the donor graft does not grow together properly with the recipient lymphatic system after transplant, resulting in lipid rich lymph fluid to leak into the abdominal cavity.
Signs and Symptoms:
 • Abdominal distention
 • Early satiety
 • Abdominal pain
 • Nausea/ vomiting
Diagnosis: Diagnosis is based on the clinical picture, observation of a milky fluid coming from JP drains, and/or from imaging studies such as an abdominal CT scan.
Treatment: Treatment is mainly supportive with NPO (nothing by mouth), fat-free diet and bowel rest. In refractory cases, surgical intervention may be necessary.

Question 12

Chronic Rejection

Accepted Answer

This type of rejection develops months to years after the transplant has been completed and after acute rejection episodes have subsided. It appears to be a combination of both antibody and cell mediated rejection. Overall, there is fibrosis and scarring of the transplanted organs.
The only way to definitively diagnose chronic rejection is through a full thickness biopsy of the graft to look at the histology. A full thickness biopsy means that an entire portion of the intestine must be removed for full examination, unlike the pin-point biopsies that can be taken during a scope procedure. This is completed in the operating room.
Risks for chronic rejection include:
 • Acute rejection within the first month of transplant.
 • Isolated small bowel graft (in many reports it has been shown that including the liver in the graft has an immunoprotective effect in preventing rejection).
 • Greater number of acute rejection episodes.
 • Older recipient age.
Symptoms of chronic rejection include:
 • Abdominal pain and/or distention.
 • Increased output from ostomy.
 • Poor oral intake and/or decreased appetite.
 • Weight loss and/or failure to thrive.

Question 13

Kidney (Renal) Dysfunction

Accepted Answer

After an intestinal transplant, your child will most likely to be on high doses of immunosuppression medications, particularly tacrolimus, which is in a class of medication known as calcineurin inhibitors. This particular class of medication is particularly hard on the kidneys and can lead to kidney dysfunction and disease.
What is the function of the kidney?
 • Filter waste materials out of the blood and pass them out of the body as urine.
 • Regulate blood pressure and the levels of water, salts, and minerals in the body.
 • Produce hormones that control other body functions.
Your child's transplant team will take blood on a consistent basis to monitor the function of his or her kidneys. Your serum creatine, BUN, and glomerular filtration rate (GFR) are indicators of kidney dysfunction.
 • Serum creatinine: This is a waste product that comes from muscle activity. When kidneys are working well, they remove creatinine from the blood.
 • Glomerular filtration rate (GFR): A math formula using the person’s age, race, gender and their serum creatinine is used to calculate a GFR. This number is used to figure out the stage of chronic kidney disease (CKD).
 • Blood Urea Nitrogen (BUN): A normal waste product in your child's blood that comes from the breakdown of protein from the foods your child eats and from his or her body's metabolism. It rises with decreased kidney function as well as when your child is dehydrated.

Post-TransplantComplications

Post-Transplant
Complications